How is Susac's syndrome treated?

Susac's syndrome is best treated as soon as possible with an aggressive, long-term course of drugs that suppress the immune system, such as steroids. Medications may include:

  • Prednisone
  • Intravenous methylprednisolone
  • Intravenous immunoglobulin (IVIG)
  • Cyclophosphamide
  • Mycophenolate mofetil
  • Azathioprine
  • Biological therapies

Some doctors recommend that Susac's syndrome patients avoid the use of oral contraceptives and estrogen-replacement therapies because hormones may aggravate the blockage of blood vessels.

What can be expected after treatment for Susac's syndrome?

The immunosuppressive drugs give the body a chance to correct itself while slowing down the immune system's attacks. As this correction takes place, the dosage of drugs can be decreased. The patient should be watched so that drug levels are kept in balance with the rate at which the immune system stabilizes. If treatment is discontinued too quickly, the disease may flare up again.

Last reviewed by a Cleveland Clinic medical professional on 05/26/2017.

References

  • Susac, JO. Editorial: Susac's Syndrome. Am J Neuroradiology 2004 25:351-352.
  • Genetic and Rare Diseases Information Center (GARD). Susac syndrome Accessed 5/26/2017.
  • Rennebohm R, Susac JO, Egan RA, Daroff RB. Susac's Syndrome--update. J Neurol Sci 2010 Dec 15;299(1-2):86-91. Epub 2010 Sep 19. PMID: 20855088 [PubMed - indexed for MEDLINE]
  • Rennebohm RM, Egan RA, Susac JO. Treatment of Susac's Syndrome. Curr Treat Options Neurol. 2008 Jan;10(1):67-74. PMID: 18325301
  • Susac JO, Egan RA, Rennebohm RM, Lubow M. Susac's syndrome: 1975-2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci 2007 Jun 15;257(1-2):270-2. Epub 2007 Feb 28. Review. PMID: 17331544 [PubMed - indexed for MEDLINE] [PubMed]
  • Rennebohm RM, Susac JO. Treatment of Susac's syndrome. J Neurol Sci 2007 Jun 15;257(1-2):215-20. Epub 2007 Feb 26. Review. PMID: 17324441 [PubMed - indexed for MEDLINE]
  • Rennebohm RM, Lubow M, Rusin J, Martin L, Grzybowski DM, Susac JO. Aggressive immunosuppressive treatment of Susac's syndrome in an adolescent: using treatment of dermatomyositis as a model. Pediatr Rheumatol Online J 2008 Jan 29;6:3. PMID: 18230188 [PubMed]

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